Background: and PurposeImpulse-control disorder is an important nonmotor symptom of Parkinson's disease (PD) that can lead to financial and social problems, and be related to a poor quality of life. A nationwide multicenter prospective study was performed with the aim of validating the Korean Version of the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease Rating Scale (K-QUIP-RS). Methods: The K-QUIP-RS was constructed using forward and backward translation, and pretesting of the prefinal version. PD patients on stable medical condition were recruited from 27 movement-disorder clinics. Participants were assessed using the K-QUIP-RS and evaluated for parkinsonian motor and nonmotor statuses and for PD-related quality of life using a predefined evaluation battery. The test–retest reliability of the K-QUIP-RS was assessed over an interval of 10–14 days, and correlations between the KQUIP-RS and other clinical scales were analyzed. Results: This study enrolled 136 patients. The internal consistency of the K-QUIP-RS was indicated by a Cronbach's α coefficient of 0.846, as was the test–retest reliability by a Guttman split-half coefficient of 0.808. The total K-QUIP-RS score was positively correlated with the scores for depression and motivation items on the Unified PD Rating Scale (UPDRS), Montgomery-Asberg Depression Scale, and Rapid-Eye-Movement Sleep-Behavior-Disorders Questionnaire. The total K-QUIP-RS score was also correlated with the scores on part II of the UPDRS and the PD Quality of Life-39 questionnaire, and the dopaminergic medication dose. Conclusions The K-QUIP-RS appears to be a reliable assessment tool for impulse-control and related behavioral disturbances in the Korean PD population.

Immunoglobulin G4-related disease is a recently recognized entity characterized by a mass-forming or regional lesion that contains an extensive infiltration of IgG4-producing plasma cells with dense fibrosis. Immunoglobulin G4-related disease can affect any organ system, but solitary hepatic lesion of Immunoglobulin G4-related disease is very rare. This entity mimics primary malignant hepatic tumor, such as hepatocellular carcinoma or intrahepatic cholangiocarcinoma. We experienced a case of hepatic IgG4-related inflammatory pseudotumor in a 50-year-old woman, mimicking hepatocellular carcinoma.
Carcinoma, Hepatocellular* ; Cholangiocarcinoma ; Female ; Fibrosis ; Granuloma, Plasma Cell* ; Humans ; Immunoglobulins* ; Middle Aged ; Plasma Cells

OBJECTIVE: To evaluate the care status of the amyotrophic lateral sclerosis (ALS) patients with long-term use of tracheostomy tube by caregivers of ALS patients. METHODS: A survey was conducted in the form of questionnaires to ALS patients and their caregivers. All measurements were performed by two visiting nurses. For statistical analysis, SPSS ver. 22.0 and Mann-Whitney U test on non-normal distribution were used. RESULTS: In total, 19 patients (15 males and 4 females) and their caregivers participated in the survey. In the case of patients, the average duration of care was 5.9+/-3.7 years, and the mean periods of illness and tracheostomy were 5.3+/-3.2 years and 3.0+/-2.6 years, respectively. Replacement intervals were 14 days in 11 patients, 7 days in 4 patients, 28 days in 2 patients, and 21 days in 1 patient. One patient was unable to provide an accurate replacement interval. Eighteen (99%) caregivers had experience of adding volume to a cuff without pressure measure in the following instances: due to patients' needs in 7 cases, air leakage in 7 cases, and no reason in 4 cases. Mean pressure of tracheostomy cuff was 40+/-9.4 cmH2O, and air volume of tracheostomy cuff was 6.7+/-3.2 mL, but real mean volume was 7.0+/-2.9 mL. The number of suctioning for airway clearance was a mean 27.5+/-18.2 times a day. CONCLUSION: According to this survey, we notice that almost all the patients and caregivers had an erroneous idea about cuff volume and pressure. Moreover, education and long-term professional care of tracheostomy cannot be overemphasized in this manner.
Amyotrophic Lateral Sclerosis ; Caregivers ; Education ; Humans ; Long-Term Care ; Male ; Nurses, Community Health ; Suction ; Tracheostomy*

OBJECTIVE: To compare the urodynamic study variables at the onset of vesicoureteral reflux (VUR) between the overactive and underactive bladders in patients with spinal cord injury who presented with VUR. METHODS: A total of 28 (13 cases of detrusor overactivity and 15 detrusor underactivity) men were enrolled. We compared the urodynamic variables between the two groups; detrusor pressure and bladder compliance, the infused volume at the onset of VUR measured on a voiding cystourethrography and cystometric capacity, maximum detrusor pressure, and bladder compliance during filling cystometry were recorded. RESULTS: At the onset of VUR, the bladder volume and compliance, except for the detrusor pressure, showed a significant difference between the two groups. The detrusor pressure, bladder volume, and bladder compliance relative to the cystometric capacity showed a significant difference between the two groups. The detrusor pressure, bladder volume, and bladder compliance at the onset of VUR relative to the cystometric bladder capacity did not show any significant difference between the two groups. CONCLUSION: There were differences in some variables at the onset of VUR depending on the type of neurogenic bladder. The VUR occurred at a lower capacity in neurogenic bladder with detrusor overactivity than in neurogenic bladder with detrusor underactivity at the same pressure. VUR occurred at a lower intravesical pressure compared to that known as the critical detrusor pressure (> or =40 cm H2O) required for the development of VUR. The results of our study demonstrate that the detrusor pressure should be maintained lower than the well known effective critical detrusor pressure for the prevention and treatment of VUR.
Compliance ; Humans ; Male ; Spinal Cord Injuries ; Urinary Bladder ; Urinary Bladder, Neurogenic* ; Urodynamics* ; Vesico-Ureteral Reflux*

Klinefelter's syndrome (KFS) is a gonosomal aberration disease that occurs in males, and is characterized by 47, XXY karyotype, hypogonadism and a lack of secondary sexual characteristics. A potential link between this hormonally deficient syndrome and autoimmune disease, particularly systemic lupus erythematosus (SLE), has been reported. On the other hand, KFS is rarely reported to be accompanied by rheumatoid arthritis (RA), and there are no Korean cases reported. We report the first Korean case of a KFS patient with sero-positive RA and discuss the role of the pathogenesis of RA with KFS.
Aluminum Hydroxide ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Carbonates ; Hand ; Humans ; Hypogonadism ; Karyotype ; Klinefelter Syndrome ; Lupus Erythematosus, Systemic ; Male ; X Chromosome

BACKGROUND/AIMS: The incidence of Hepatitis B has significantly declined since the introduction of an HBV vaccination program. The aim of this study was to investigate recent clinical features of acute viral hepatitis B (AVH-B) in Korea. METHODS: A total of 2241 patients with acute viral hepatitis were enrolled and their data were collected from nine medical-centers between January 2006 and December 2009. RESULTS: One hundred nineteen (5.3%) of the 2241 were diagnosed as AVH-B. Among 78 patients with AVH-B whose data were analyzed, 50 were male, and the mean age was 38.6 years. In an initial test, mean AST, ALT and total-bilirubin levels were 1296.2 IU/L, 2109.6 IU/L and 9.3 mg/dl, respectively. Positivity frequencies for HBeAg and anti-HBe were 55.1% and 67.9%, respectively, and the mean HBV DNA level was 5.2 log10 copies/ml. The mean length of hospitalization was 11.6 days. During follow-up, AST, ALT and total bilirubin levels were normalized or near-normalized in all patients without serious complications. Sixty-three of 66 (95.4%) patients showed HBsAg loss and 37 (56.1%) patients showed HBsAg seroconversion. Only 3 patients (4.5%) showed persistent hepatitis B viremia. There was no case of death or liver transplantation. Nine patients (11.3%) had received anti-viral agents and their clinical outcomes were not significantly different from those of patients treated without antiviral agents. CONCLUSIONS: The prevalence of AVH-B among acute hepatitis patients is relatively low in Korea. AVH-B infection can be cured without complications in almost all patients, regardless of antiviral treatment.
Acute Disease ; Adult ; Alanine Transaminase/blood ; Antiviral Agents/therapeutic use ; Aspartate Aminotransferases/blood ; Bilirubin/blood ; DNA, Viral/analysis ; Female ; Hepatitis B/*diagnosis/drug therapy/epidemiology ; Hepatitis B Antibodies/blood ; Hepatitis B e Antigens/blood/immunology ; Humans ; Male ; Middle Aged ; Retrospective Studies

BACKGROUND: Leukemic cells originate from hypoxic bone marrow, which protects them from anti-cancer drugs. Although many factors that cause drug resistance in leukemic cells have been studied, the effect of hypoxia on drug-induced apoptosis is still poorly understood. METHODS: In this study, we examined the effect of hypoxia on anti-leukemic drug resistance in leukemic cell lines treated with cobalt chloride (CoCl2), a hypoxia-mimetic agent. Cellular proliferation was evaluated using the methyl thiazolyl tetrazolium (MTT) assay. Flow cytometry analysis and western blots were performed to investigate apoptosis-related proteins. RESULTS: Unlike its previously known apoptotic effect, the expression of HIF-1alpha increased the survival rate of human promyelocytic leukemia HL-60 cells when these cells were exposed to anti-leukemic drugs; these effects were mediated by heat-shock protein HSP70 and the pro-apoptotic protein Bax. CONCLUSION: These findings may provide new insights for understanding the mechanisms underlying hypoxia and for designing new therapeutic strategies for acute myeloid leukemia.
Anoxia ; Apoptosis ; Arsenicals ; Blotting, Western ; Bone Marrow ; Cell Line ; Cell Proliferation ; Cobalt ; Drug Resistance ; Flow Cytometry ; Heat-Shock Proteins ; HL-60 Cells ; Humans ; Leukemia ; Leukemia, Myeloid, Acute ; Oxides ; Proteins ; Survival Rate

BACKGROUND/AIMS: Our study aimed to evaluate the efficacy of MMF as compared with intravenous cyclophosphamide as induction therapy for proliferative lupus nephritis in Koreans. METHODS: Forty-three patients who were diagnosed with proliferative lupus nephritis (WHO Class III and IV) between Jan 2000 and Dec 2006 were included in this study. Nineteen patients were treated with oral MMF (initial dose: 1.0 g/day and then it was increased to 2.0 g/day) and 24 patients were treated with 0.75-1.0 g/m2 of monthly intravenous cyclophosphamide (CP) followed by subsequent treatment with oral corticosteroid (initial dose 1 mg/kg/day and then it was slowly tapered down) for 6 months. The demographic and laboratory findings, the response rate and the adverse events were reviewed retrospectively and these were compared between the two groups. RESULTS: A complete response occurred in 7 out of the 19 patients (36.8%) treated with MMF and in 8 out of the 24 patients (33.3%) treated with CP, and the difference was not significantly different between the two groups (p=0.66). A partial response was achieved in 52.6% and 45.8%, respectively. There were no significant differences of the laboratory findings such as serum albumin, C3, C4, the urine protein/creatinine ratio and serum creatinine after treatment for 6 months. In addition, both groups had similar rates of adverse events. CONCLUSIONS: Our study showed that for the treatment of lupus nephritis, MMF was as effective as IV cyclophosphamide with similar adverse events. This finding suggests that MMF could be an alternative treatment for active lupus nephritis as induction therapy.
Creatinine ; Cyclophosphamide ; Humans ; Lupus Nephritis ; Mycophenolic Acid ; Retrospective Studies ; Serum Albumin

OBJECTIVE: Deep vein thrombosis (DVT) is one of the most common complications of acute spinal cord injury (SCI). It is well known that the incidence of DVT in post-acute SCI patients decreases to a level similar to that in the general population. The aim of this study was to evaluate the blood coagulation status and the possibility of DVT occurrence in post-acute SCI patients. METHOD: Twenty-three SCI patients (SCI group) were included in this study. Ten patients having spinal fracture with no evidence of SCI were used as the control group. Coagulation status was examined using factor VIII antigen, factor VIII procoagulant, fibrinogen, D-dimer, protein C, and protein S in both groups. Duplex ultrasonography was performed for the diagnosis of DVT. RESULTS: Prevalence of the abnormally decreased protein S level was significantly high in the SCI group (87%) than in the control group (30%). Prevalence of the abnormally increased F VIII:Ag, F VIII:C, and fibrinogen levels in the SCI group were significantly high in the SCI group (p<0.05). CONCLUSION: We conclude that post-acute SCI patients may have hypercoagulability. Proper physical prevention and thromboprophylaxis should be considered in post-acute SCI patients because they have relatively higher risk of thromboembolic complication.
Blood Coagulation ; Diagnosis ; Factor VIII ; Fibrinogen ; Humans ; Incidence ; Prevalence ; Protein C ; Protein S ; Spinal Cord Injuries ; Spinal Cord* ; Spinal Fractures ; Thrombophilia ; Ultrasonography ; Venous Thrombosis

BACKGROUND: Although high dose chemotherapy coupled with an autologous stem cell transplantation (ASCT) is widely accepted as effective therapy for multiple myeloma (MM), few reports are available in Korea, especially in the area of double ASCT. We present the results of an institutional retrospective study of 12 patients with MM treated by double ASCT. METHODS: Eligible patients received induction therapy using vincristine, adriamycin, dexamethasone (VAD), and mobilization was performed using cyclophosphamide plus lenograstim. High-dose melphalan (total 200 mg/m2) was used to condition the ASCT. RESULTS: The median interval from diagnosis to ASCT was 6 months (range, 1.8-15.3 months). The median interval between the 1st and 2nd ASCT was 4.4 months (range 2.1-48.7 months). The median follow up was 18.3 months (range 8.1-50.5 months) for the nine surviving patients. No therapy-related mortality occurred. Following induction chemotherapy, two patients experienced CR. Following double ASCT, eight patients experienced CR. The 5 year OS was 59%. The median duration of event free survival was 2.13 years (95% CI, 0.84-3.42). CONCLUSION: Although the results of study did not demonstrate the advantage of double ASCT, this is the first report to outline the outcome of double ASCT for Korean MM patients.
Vincristine/administration & dosage ; Transplantation, Autologous ; *Stem Cell Transplantation ; Retrospective Studies ; Recombinant Proteins/administration & dosage ; Multiple Myeloma/drug therapy/*therapy ; Middle Aged ; Male ; Korea ; Humans ; Granulocyte Colony-Stimulating Factor/administration & dosage ; Female ; Doxorubicin/administration & dosage ; Dexamethasone/administration & dosage ; Antineoplastic Combined Chemotherapy Protocols/*administration & dosage ; Antineoplastic Agents/*therapeutic use ; Aged ; Adult